多数のtangleを伴ったargyrophilic grain dementiaの1剖検例

An Autopsy Case of Argyrophilic Grain Dementia with Abundant Neurofibrillary Tangles

要旨　70歳時に発症し，全経過10年で，その神経病理学的所見がargyrophilic grain dementiaともtangle only dementiaともつく症例を報告した。記銘力障害にて発症し，最終的には10年後に誤嚥性肺炎を繰り返し，衰弱して死去した。病理学的検討では，脳重は1,220g，肉眼的に軽度の側頭葉の萎縮を認めた。組織学的には海馬傍回，扁桃核皮質内側群，Meynert基底核，青斑核，黒質に神経原線維変化の出現を認め（Braak stage：V），島回，扁桃核皮質内側群，扁桃核基底外側群，T3，T4にargyrophilic grainの出現を認めた。また扁桃核や海馬傍回にballooned neuronの出現を認めた。老人斑はT4に一部限局し少量認めるのみであった（Braak stage：A）。T4領域より抽出したサルコシル不溶性タウは，主に4リピートタウで構成されていた。

　We reported an autopsy case of “senile dementia" showing neuropathologically abundant neurofibrillary tangles（NFT） and argyrophilic grains（AG） without senile plaques. A Japanese woman developed memory disturbance when she was 70 years old. The patient was hospitalized at age 80 and a cranial CT scan revealed bilateral mild atrophy of the temporal lobes and mild enlargement of the lateral ventricle, especially in the inferior horn. She died at the age of 80. Autopsy showed that her brain weighted 1220g. Numerous NFT were found in the entorhinal（trans-entorhinal） region, subiculm, CA1-CA4, dentate gyrus, amygdala, nucleus basalis of Meynert, substantia nigra, and locus coeruleus. Furthermore, numerous AG were seen in the temporal lobe（T3, T4）,a mygdala, prominently in the basolateral nuclei. Obvious neuronal loss with gliosis was noted in the temporal lobes, including the hippocampal regions. Few senile plaques was detected in temporal lobe（T4）. Sarkosyl-insoluble tau extracted from the temporal lobe consisted predominantly of four-repeat tau isoforms. To our knowledge, this is the first report of argyrophilic grain dementia complicated with tangle only dementia.